Neurosarcoidosis: a clinical approach to diagnosis and management
File(s)
Author(s)
Ibitoye, RT
Wilkins, A
Scolding, NJ
Type
Journal Article
Abstract
Sarcoidosis is a rare but important cause of
neurological morbidity, and neurological symptoms often
herald the diagnosis. Our understanding of neurosarcoidosis
has evolved from early descriptions of a uveoparotid
fever to include presentations involving every part of the
neural axis. The diagnosis should be suspected in patients
with sarcoidosis who develop new neurological symptoms,
those presenting with syndromes highly suggestive of
neurosarcoidosis, or neuro-inflammatory disease where
more common causes have been excluded. Investigation
should look for evidence of neuro-inflammation, best
achieved by contrast-enhanced brain magnetic resonance
imaging and cerebrospinal fluid analysis. Evidence of
sarcoidosis outside the nervous system should be sought in
search of tissue for biopsy. Skin lesions should be identi-
fied and biopsies taken. Chest radiography including highresolution
computed tomography is often informative. In
difficult cases, fluorodeoxyglucose positron emission
tomography and gallium-67 imaging may identify subclinical
disease and a target for biopsy. Symptomatic
patients should be treated with corticosteroids, and if
clinically indicated other immunosuppressants such as
hydroxychloroquine, azathioprine, cyclophosphamide or
methotrexate should be added. Anti-tumour necrosis factor
alpha therapies may be considered in refractory disease but
caution should be exercised as there is evidence to suggest
they may unmask disease.
neurological morbidity, and neurological symptoms often
herald the diagnosis. Our understanding of neurosarcoidosis
has evolved from early descriptions of a uveoparotid
fever to include presentations involving every part of the
neural axis. The diagnosis should be suspected in patients
with sarcoidosis who develop new neurological symptoms,
those presenting with syndromes highly suggestive of
neurosarcoidosis, or neuro-inflammatory disease where
more common causes have been excluded. Investigation
should look for evidence of neuro-inflammation, best
achieved by contrast-enhanced brain magnetic resonance
imaging and cerebrospinal fluid analysis. Evidence of
sarcoidosis outside the nervous system should be sought in
search of tissue for biopsy. Skin lesions should be identi-
fied and biopsies taken. Chest radiography including highresolution
computed tomography is often informative. In
difficult cases, fluorodeoxyglucose positron emission
tomography and gallium-67 imaging may identify subclinical
disease and a target for biopsy. Symptomatic
patients should be treated with corticosteroids, and if
clinically indicated other immunosuppressants such as
hydroxychloroquine, azathioprine, cyclophosphamide or
methotrexate should be added. Anti-tumour necrosis factor
alpha therapies may be considered in refractory disease but
caution should be exercised as there is evidence to suggest
they may unmask disease.
Date Issued
2016-11-22
Date Acceptance
2016-11-07
Citation
Journal of Neurology, 2016, 264 (5), pp.1023-1028
ISSN
0340-5354
Publisher
Springer Verlag
Start Page
1023
End Page
1028
Journal / Book Title
Journal of Neurology
Volume
264
Issue
5
Copyright Statement
© The Author(s) 2016. This article is published with open access at Springerlink.com
License URL
Subjects
Science & Technology
Life Sciences & Biomedicine
Clinical Neurology
Neurosciences & Neurology
Sarcoidosis
Neurosarcoidosis
Diagnosis
Treatment
Management
NERVOUS-SYSTEM SARCOIDOSIS
THERAPY
MANIFESTATIONS
INFLIXIMAB
SERIES
SIGNS
Publication Status
Published